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Annals of Hematology

, Volume 98, Issue 10, pp 2273–2281 | Cite as

Clinical implication of renal dysfunction during the clinical course in patients with paroxysmal nocturnal hemoglobinuria: a longitudinal analysis

  • Jin Seok KimEmail author
  • June-Won Cheong
  • Yeung-Chul Mun
  • Jun Ho Jang
  • Deog-Yeon Jo
  • Jong Wook LeeEmail author
  • on behalf of Aplastic Anemia Working Party of the Korean Society of Hematology
Original Article

Abstract

Although renal dysfunction at the time of diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is a risk factor for mortality, subsequent renal events can occur. The objective of this study was to identify clinical implication of renal dysfunction occurring during the disease course in PNH patients. One hundred one patients with a granulocyte clone size of > 10% were enrolled. Renal events were observed in 55 (54.5%) patients during a median follow-up of 94.2 months. Median time to first renal event from diagnosis of PNH was 79.3 months. Thromboembolism (TE) event and recurrent TE events were observed in 25 (24.8%) and 8 (7.9%) patients, respectively. The rate of recurrent TE was significantly higher in patients with renal events ≥ 2 compared with that in patients with renal event ≤ 1 (18.8% vs. 2.9%; P = 0.012). The rate of recurrent TE was significantly higher in patients with chronic kidney disease (CKD) + acute kidney disease (AKD) compared with the rest of the patients (27.3% vs. 5.6%; P = 0.040). CKD+AKD was the only independent risk factor for OS in multivariate analysis (hazard ratio 7.95, 95% CI 1.24–51.15, P = 0.029). Therefore, close monitoring of renal events in PNH patients during the entire clinical course is essential.

Keywords

Paroxysmal nocturnal hemoglobinuria Renal dysfunction Thromboembolism Mortality 

Notes

Funding information

This work was supported by the Korean Society of Hematology.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interests.

Ethical compliance

All the authors stated that the study have been approved by the appropriate institutional review board and have been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. Since this is a retrospective study, formal informed consent is not required.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Jin Seok Kim
    • 1
    Email author
  • June-Won Cheong
    • 1
  • Yeung-Chul Mun
    • 2
  • Jun Ho Jang
    • 3
  • Deog-Yeon Jo
    • 4
  • Jong Wook Lee
    • 5
    Email author
  • on behalf of Aplastic Anemia Working Party of the Korean Society of Hematology
  1. 1.Division of Hematology, Department of Internal Medicine, Severance HospitalYonsei University College of MedicineSeoulRepublic of Korea
  2. 2.Division of Hematology and Oncology, Department of Internal Medicine, Collage of MedicineEwha Womans UniversitySeoulRepublic of Korea
  3. 3.Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, School of MedicineSungkyunkwan UniversitySeoulRepublic of Korea
  4. 4.Department of Internal Medicine, School of Medicine, Chungnam National University HospitalChungnam National UniversityDaejeonRepublic of Korea
  5. 5.Division of Hematology, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of MedicineThe Catholic University of KoreaSeoulRepublic of Korea

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